아급성 경화성 범뇌염(Subacute Sclerosing Panencephalitits) 1례

A Case of Subacute Sclerosing Panencephalitis

저자들은 5개월 때 홍역을 앓은 과거력이 있으면서 5세까지 정상적인 발달과 성장을 보이다 근간대성 경련이 생기면서 2개월만에 발달 장애, 정신지체, 기면상태를 보이는 남아에서 특징적인 뇌파 소견과 뇌척수액내 홍역 바이러스 항체 역가의 상승 등으로 진단된 아급성 경화성 범뇌염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다

Subacute sclerosing panencephalitis(SSPE) is a progressive inflammatory disease of the central nervous system(CNS) caused by a persistent, aberrant measles virus infection. The outcome is usually fatal. After a latent period of 6 to 7 years, there is subtle and slow cognitive decline and visuospatial disorientation develops followed by myoclonic jerks, extrapyramidal symptoms, ataxia, and seizures, progressing to coma or vegetative state. The diagnosis is based on at least three of the following criteria 1) clinical manifestations 2) abnormal EEG 3) hyperglobulinorrachia, elevated serum or spinal fluid measles antibody 4) histologic features. No therapeutic maneuver has been proven conclusively to be of value. We have diagnosed and experienced a case of subacute sclerosing panencephalitis(SSPE) in a 5-year-old child with the chief complaint of myoclonic seizure and mental deterioration. We report a case and the brief review of related literature.